DOI:10.2214/07.2104
AJR 2007; 189:1353-1360
© American Roentgen Ray Society
Postoperative Imaging in Cyanotic Congenital Heart Diseases: Part 1, Normal Findings
Esther Rodríguez1,
Rafaela Soler1,
Rosa Fernández1 and
Inés Raposo2
1 Department of Radiology, Complejo Hospitalario Universitario Juan Canalejo,
Xubias de Arriba 84, 15006 La Coruña, Spain.
2 Department of Pediatric Cardiology, Complejo Hospitalario Universitario Juan
Canalejo, La Coruña, Spain.
Received February 23, 2007;
revised June 19, 2007;
Address correspondence to E. Rodríguez
(esther.rodriguez{at}mundo-r.com).
CME
This article is available for CME credit. See
www.arrs.org
for more information.
Abstract
OBJECTIVE. The objective of this article is to illustrate the most
common surgical procedures performed in patients with cyanotic congenital
heart diseases along with the respective postoperative MRI findings normally
seen in clinical practice.
CONCLUSION. Radiologists need a solid knowledge of the surgical
procedures used to treat patients with cyanotic congenital heart diseases to
identify what constitutes normal postoperative findings on MR images and to
play an ongoing role in the integral lifelong care of these patients.
Keywords: cardiac surgery • cine imaging • congenital heart disease • cyanosis • heart disease • hemodynamics • MR angiography • MRI • shunts
Introduction
Improvements in surgical techniques and medical treatment over the past two
decades have increased the life span of patients with cyanotic congenital
heart diseases more than ever. Cardiac MRI is an ideal technique for
evaluating postsurgical morphology and function in these patients
[1]. Contrast-enhanced 3D MR
angiography (MRA), in turn, can be used to effectively assess the extracardiac
aspects of surgery.
An understanding of the surgical procedures used to treat patients with
cyanotic congenital heart diseases and of their postoperative appearances on
MR images is a basic requirement for radiologists to be able to differentiate
normal postoperative findings from complications. This article, which includes
sketches of the most common surgical palliation and repair procedures
performed in patients with cyanotic congenital heart diseases, aims to
contribute to that understanding.
Extracardiac Procedures
Systemic Arterial–to–Pulmonary Artery Shunts
Systemic arterial–to–pulmonary artery shunts (i.e.,
Blalock-Taussig, Potts, Waterston-Cooley, Davidson, and Sano shunts) are
palliative surgical procedures performed to increase the delivery of
desaturated venous blood to the lungs, thereby alleviating cyanosis and
enlarging the pulmonary arteries.
Blalock-Taussig shunt—The subclavian
artery–to–pulmonary artery shunt or Blalock-Taussig shunt
(Fig. 1A) used in the past has
been largely replaced by the modified Blalock-Taussig shunt in which a graft
connects the subclavian artery to the pulmonary artery
[2]
(Fig. 1B). The advantages of
the modified Blalock-Taussig shunt over the standard Blalock-Taussig shunt
include greater growth of the pulmonary tree and less distortion of the
pulmonary arteries.
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Fig. 1A —Blalock-Taussig shunt. Sketch of Blalock-Taussig shunt.
Drawing shows classic Blalock-Taussig procedure in which end-to-side
anastomosis (gray) is performed between subclavian artery and
ipsilateral pulmonary artery, usually on side opposite descending aorta.
Although this procedure provides shunt flow appropriate for patient who is
size of infant, it requires careful, lengthy dissection and distorts
peripheral pulmonary artery.
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Fig. 1B —Blalock-Taussig shunt. Sketch shows modified Blalock-Taussig
shunt, in which prosthetic graft material (gray) is inserted between
subclavian artery and ipsilateral pulmonary artery. With this modified shunt,
which can be performed on either side, subclavian blood supply to arm is
preserved.
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MRI is a robust technique for visualizing shunt patency
(Fig. 1C) and the increase in
size of the pulmonary arteries after the procedure
[1].
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Fig. 1C —Blalock-Taussig shunt. Oblique coronal thin-slab reformatted
maximum-intensity-projection image obtained with gadolinium-enhanced 3D MR
angiography shows patent modified Blalock-Taussig shunt (arrows) from
right subclavian artery to pulmonary artery. Procedure was performed for
palliative correction of tetralogy of Fallot in 6-year-old boy.
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Potts, Waterston-Cooley, and Davidson shunts—A Potts shunt
consists of creating a small communication between the posterior wall of the
left pulmonary artery and the anterior aspect of the ipsilateral descending
thoracic aorta (Fig. 2A). In
the Waterston-Cooley shunt, the small communication is created between the
posterior wall of the ascending aorta and the anterior wall of the right
pulmonary artery (Fig. 2B).
Potts and Waterston-Cooley shunts have become obsolete because of the high
incidence of pulmonary hypertension and distortion of the pulmonary arteries
recorded [2].
In the Davidson shunt, also called a "central shunt," a
prosthetic graft material is inserted between the ascending aorta and the main
pulmonary artery (Fig. 2C).
This shunt is usually performed when the pulmonary arteries are
hypoplastic.
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Fig. 2C —Aortopulmonary shunt. Sketch shows central shunt, in which
prosthetic graft material (gray) is inserted between ascending aorta
and main pulmonary artery. Amount of shunt flow is controlled by size of graft
(usually 4–5 mm in diameter). This procedure prevents distortion of
pulmonary arteries and allows symmetric blood flow and growth.
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Sano shunts—Today, many cardiac surgeons use a Sano shunt in
which an extracardiac allograft valved conduit is inserted directly from the
right ventricle to the pulmonary artery (Figs.
2D and
2E). This shunt is created to
avoid the reduced diastolic blood flow in the coronary circulation associated
with the Blalock-Taussig shunt
[3].
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Fig. 2D —Aortopulmonary shunt. Sketch of Sano shunt shows from right
ventricle to pulmonary bifurcation using prosthetic graft conduit
(gray). Important advantage of Sano shunt is that flow occurs only
during systole. There is no competition between pulmonary and coronary blood
flow during diastole, as is case with Blalock shunt.
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Fig. 2E —Aortopulmonary shunt. Oblique coronal
maximum-intensity-projection image shows Sano shunt from right ventricle
(arrow) to pulmonary artery (arrowhead) performed for
palliative correction of pulmonary atresia in 4-year-old boy.
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Systemic Venous–to–Pulmonary Artery Shunts
Systemic venous–to–pulmonary artery shunts (i.e., Glenn,
Fontan, and Rastelli procedures) provide venous flow to the lung fields for
oxygenation without the increase in ventricular workload or volume observed in
systemic arterial–to–pulmonary artery shunts.
Glenn shunt—The original Glenn shunt, consisting of an
anastomosis of the superior vena cava (SVC) and the distal end of the divided
right pulmonary artery, provided perfusion of only the right lung. This
technique has been largely replaced today. The modified procedure that is in
current use is termed the "bidirectional Glenn shunt," in which an
end-to-side anastomosis of the SVC and the right pulmonary artery provides
bidirectional flow to the lungs without raising the workload on the heart
(Fig. 3A,
3B,
3C).
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Fig. 3A —Modified Glenn shunt. Sketch of bidirectional Glenn shunt.
Diagram depicts postoperative anatomy of bidirectional Glenn shunt
(gray) in which superior vena cava (SVC) is disconnected from right
atrium and anastomosed to undivided right pulmonary artery, providing flow for
both lung fields. As with classic Glenn shunt, bidirectional cavopulmonary
shunt is less likely to engender pulmonary vascular obstructive disease than
systemic artery–to–pulmonary artery shunts and involves only
minimal distortion of pulmonary artery architecture.
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Fig. 3B —Modified Glenn shunt. Postoperative anterior 3D shaded
surface display (B) and maximum-intensity-projection (C) images
show bidirectional Glenn shunt extending from SVC (arrows) to right
pulmonary artery (stars) performed for tricuspid atresia and proximal
right pulmonary artery stenosis in 20-year-old man. Bright blue and white show
SVC and right pulmonary artery, respectively, in C.
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Fig. 3C —Modified Glenn shunt. Postoperative anterior 3D shaded
surface display (B) and maximum-intensity-projection (C) images
show bidirectional Glenn shunt extending from SVC (arrows) to right
pulmonary artery (stars) performed for tricuspid atresia and proximal
right pulmonary artery stenosis in 20-year-old man. Bright blue and white show
SVC and right pulmonary artery, respectively, in C.
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Depending on the diagnosis and timing of the surgery, the Glenn shunt may
be only one of several palliative procedures for a cyanotic patient, a step in
palliative surgery for a total right heart bypass with the Fontan procedure,
which requires an intact, unobstructed pulmonary artery tree
[4].
Fontan circulation—The aim of the Fontan procedure is to
establish circulation in which the systemic venous return enters the pulmonary
arteries directly [4]. The
original Fontan operation consisted of placing a valved conduit between the
right atrium or atrial appendage and the pulmonary artery (Figs.
4A and
4B). There are many procedural
variations, including the direct right atrium–right ventricle
(Björk modification) (Fig.
4C) and total cavopulmonary connections, the latter consisting of
either an intraatrial tunnel (Fig.
4D) or an extracardiac conduit (Figs.
4E,
4F,
4G). A small residual atrial
shunt (fenestrated Fontan) may be deliberately constructed to moderate
systemic venous pressure in the postoperative period
(Fig. 4H).
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Fig. 4B —Fontan procedures. Contrast-enhanced 3D reformatted
volume-rendered MR angiography image shows hypoplastic right ventricle
(star), large right atrium (arrows), and patent conduit
(arrowhead) between right atrial appendage and right pulmonary
artery, as in original Fontan procedure, in 12-year-old boy. Yellow shows
conduit, blue shows right atrium and right ventricle, and red shows left
atrium and left ventricle.
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Fig. 4D —Fontan procedures. Sketch of lateral tunnel procedure.
Diagram shows postoperative anatomy of intraatrial tunnel (lateral tunnel
procedure). Baffle in right atrium directs inferior vena cava (IVC) flow to
lower portion of divided superior vena cava (SVC), which is connected to
pulmonary artery. Upper part of SVC is connected to superior aspect of
pulmonary artery as in bidirectional Glenn shunts. Right and left pulmonary
arteries are interconnected, while pulmonary trunk is disconnected from heart.
Most of right atrium is excluded from systemic venous circuit. Gray area shows
right atrial baffle connected to right pulmonary artery and IVC, pulmonary
trunk disconnected from heart, and SVC to right pulmonary artery
anastomosis.
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Fig. 4E —Fontan procedures. Sketch shows extracardiac Fontan
procedure, in which IVC blood is directed to pulmonary artery via extracardiac
conduit. SVC is anastomosed to pulmonary artery, as in modified Glenn shunt,
and pulmonary trunk is disconnected from heart. Gray area shows extracardiac
conduit connected to right pulmonary artery and IVC, pulmonary trunk
disconnected from heart, and SVC to right pulmonary artery anastomosis.
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Fig. 4F —Fontan procedures. Reconstructed shaded surface display
gadolinium-enhanced 3D MR angiography (F) and coronal cine MR
(G) images in 22-year-old woman reveal patency of extracardiac conduit
(stars; yellow in F) between IVC and right pulmonary
artery (stars) as well as anastomosis of SVC and proximal right
pulmonary artery stenosis (arrows, F), as in modified
extracardiac Fontan procedure.
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Fig. 4G —Fontan procedures. Reconstructed shaded surface display
gadolinium-enhanced 3D MR angiography (F) and coronal cine MR
(G) images in 22-year-old woman reveal patency of extracardiac conduit
(stars; yellow in F) between IVC and right pulmonary
artery (stars) as well as anastomosis of SVC and proximal right
pulmonary artery stenosis (arrows, F), as in modified
extracardiac Fontan procedure.
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Fig. 4H —Fontan procedures. Sketch shows fenestrated Fontan, in which
surgical creation of atrial septal defect in atrial patch or baffle
(gray) provides escape valve, allowing right-to-left shunting to
reduce pressure in systemic venous circuit, with attendant systemic hypoxemia.
This fenestration either closes spontaneously or is occluded by device in due
course.
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The Fontan circuit has now been extended to palliate most forms of a single
functional ventricle. Postoperative ventricular function, the Fontan
circulation itself, and the dimensions of the right and left pulmonary
arteries expressed as the McGoon ratio—that is, the sum of the diameters
of the two central pulmonary arteries immediately upstream of where they
branch divided by the diameter of the descending aorta measured just above the
diaphragm—should be carefully monitored by cine MRI and
gadolinium-enhanced 3D MRA. Increased risk of death or a dysfunctional Fontan
circuit may be expected when this ratio is less than 1.8
[5].
Rastelli procedure—In the Rastelli procedure, blood is
redirected at the ventricular level with a baffle in the right ventricle to
connect the left ventricle to the aorta and a valved conduit from the right
ventricle to the pulmonary artery (Fig.
5A,
5B). Because it was the first
operation that incorporated a systemic ventricle to repair dextroposed
transposition of the great arteries (D-TGA), it was regarded as an anatomic
correction. Actually, however, it is a palliative procedure given that the
patient is committed to additional operations because the conduit is likely to
need to be replaced several times during the patient's life.
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Fig. 5A —Rastelli procedure. Drawing depicts Rastelli procedure
postoperative anatomy. In this procedure, prosthetic tunnel (gray) is
constructed from left ventricle to aorta through ventricular septal defect.
Continuity between right ventricle and main pulmonary artery is restored with
extracardiac conduit.
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Fig. 5B —Rastelli procedure. Contrast-enhanced 3D shaded surface
display MR angiography oblique coronal image shows conduit (yellow)
between right ventricle and main pulmonary artery of Rastelli operation
performed for dextrotransposition of great arteries in 28-year-old man with
ventricular septal defect and left ventricular outflow tract obstruction.
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Nowadays, the Rastelli operation is relegated to alternative status in
cases of D-TGA when the membrane obstructing left ventricular outflow is not
resectable during the anatomic correction
[6].
Pulmonary Artery Banding
Surgical stenosis of the main pulmonary artery, pulmonary artery banding
(Fig. 6A), is widely used by
cardiac surgeons worldwide in patients with cyanotic congenital heart diseases
and excessive pulmonary blood flow to protect the pulmonary vasculature from
hypertrophy and irreversible pulmonary hypertension. The current indications
for banding include multiple ventricular septal defects (Swiss-cheese
interventricular septum), ventricular septal defect with coarctation of the
aorta, and D-TGA in patients who are not immediate candidates for switch
procedure [7]. In D-TGA, the
band is placed to increase work of the left ventricle and cause an increase in
muscle mass before corrective surgery. The anatomic position of the pulmonary
artery band (Fig. 6B), the
mass and function of the right ventricle, and the appearance of the pulmonary
vascular tree can be readily monitored using MRI.
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Fig. 6A —Pulmonary artery banding. Sketch of pulmonary artery banding.
Diagram shows surgical ligature around midportion of main pulmonary artery
(gray) causing artificial pulmonary stenosis to reduce systolic
pulmonary artery pressure. Repeated progressive occlusion and reopening are
possible with new surgically implantable devices that actually behave like
adjustable pulmonary artery bands.
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Fig. 6B —Pulmonary artery banding. Reformatted shaded surface display
contrast-enhanced 3D MR angiography image depicts correct placement of
pulmonary artery band in midportion of main pulmonary artery
(arrowhead); procedure was performed for palliative correction of
double-outlet right ventricle with large ventricular septal defect in
15-year-old girl. Blue shows right ventricle; yellow shows pulmonary artery
banding; and red shows left ventricle, pulmonary veins, and aorta.
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Intracardiac Procedures
Anatomic Repair of Tetralogy of Fallot
Reparative surgery for tetralogy of Fallot is usually performed in the
first year of life. This procedure consists of patch closure of the
ventricular septal defect and widening of the right ventricular outflow (Figs.
7A,
7B,
7C). The latter is achieved by
either removing the obstructing muscle or pulmonary valve and using a patch to
enlarge the area as needed or building a conduit from the right ventricle to
the main pulmonary artery [8].
MRI is an excellent noninvasive technique to closely monitor biventricular
function, to assess the pulmonary vascular tree
(Fig. 7D), and to detect early
and late postoperative complications.
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Fig. 7A —Complete surgical correction for tetralogy of Fallot. Sketch
of corrective surgery for tetralogy of Fallot. Diagram shows closing of
ventricular septal defect and widening of right ventricular outflow tract with
patching of infundibular tract (gray).
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Fig. 7B —Complete surgical correction for tetralogy of Fallot. Bulge
(arrowhead) visible on this axial cine MR image obtained during
diastole is ventricular septal defect closed. Defect was closed during
corrective surgery for tetralogy of Fallot in 20-year-old man. No residual
left-to-right shunt was found in phase velocity mapping images (not
shown).
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Fig. 7C —Complete surgical correction for tetralogy of Fallot. Bulge
(arrow) of right ventricular outflow tract and of main pulmonary
artery seen in this short-axis cine MR image of same patient shown in B
is patch.
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Fig. 7D —Complete surgical correction for tetralogy of Fallot.
Reformatted 3D shaded surface display MR coronal image of same patient shown
in B and C shows enlarged right ventricular outflow tract
(star) and pulmonary artery branches in which no significant
narrowing or stenosis is visible after complete surgical repair of tetralogy
of Fallot. Blue shows right atrium, right ventricle, and pulmonary arteries;
and red shows right aortic arch and apex of left ventricle.
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Physiologic Correction of D-TGA
In the physiologic correction of transposed great arteries (atrial switch)
[9], systemic venous blood from
the SVC and inferior vena cava (IVC) is redirected to the left ventricle, and
pulmonary venous blood from the pulmonary veins is redirected to the right
ventricle (Fig. 8A) using
artificial pericardial (Mustard procedure) or atrial (Senning procedure)
tissue. Although a physiologic correction circulation is created, normal
anatomic relations are not restored, and the right ventricle remains subject
to systemic loading, followed by compensatory hypertrophy. This can result in
late right ventricle failure.
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Fig. 8A —Atrial switch procedure. Sketch of atrial switch procedure.
Diagram depicts postoperative status. Systemic venous flow is directed behind
baffle into left atrium, through mitral valve, and out pulmonary artery to
lungs. Pulmonary venous return is directed over baffle, into right atrium,
through tricuspid valve, and out aorta. Gray area highlights left atrial
baffle.
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Right and left ventricular function and venous pathway patency can be
effectively evaluated with gradient-echo MRI (Figs.
8B and
8C).
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Fig. 8B —Atrial switch procedure. Axial cine MR image obtained during
diastole in 20-year-old man shows how atrial baffle (arrow) of
Senning procedure for dextrotransposition of great arteries isolates mitral
valve from pulmonary venous drainage. Pulmonary venous blood enters posterior
pulmonary venous atrium and flows anteriorly across tricuspid valve
(arrowhead). Note thickness of right ventricle wall.
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Fig. 8C —Atrial switch procedure. Coronal cine MR image obtained
during diastole in same patient shown in B shows that superior
(arrowhead) and inferior (arrow) venae cavae drain into
systemic venous baffle (star).
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Anatomic Repair of D-TGA
Today, arterial switch is the surgical treatment of choice in neonates with
D-TGA [9]. In this procedure,
surgical repair involves the repositioning of both the aorta and the pulmonary
arteries (Fig. 9A).
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Fig. 9A —Arterial switch operation (Jatene arterial switch procedure).
Anatomic sketch after arterial switch operation (Jatene arterial switch
procedure). This procedure consists of removing great vessels from their
native ventricles and switching them to contralateral ventricles, with
reimplantation of coronary arteries into neoaorta (gray). So-called
Lecompte maneuver is performed to bring branch pulmonary arteries from their
original posterior position to position anterior to aorta.
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The arterial switch procedure prevents the development of right ventricle
failure because the left ventricle is the systemic and the right, the
pulmonary ventricle. The postoperative anatomic relationship between the
ascending aorta and the pulmonary artery branches is clearly visible with 3D
gadolinium-enhanced MRA (Fig.
9B). Hemodynamic changes in the pulmonary arteries after the
arterial switch procedure (Figs.
9C and
9D) can be seen with cine MRI,
and velocity mapping can be used to evaluate the hemodynamic significance of
such changes [10]. The
reversal in muscle thickness and shape of the right ventricle can be viewed
with cine MRI.
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Fig. 9B —Arterial switch operation (Jatene arterial switch procedure).
Reconstructed shaded surface display gadolinium-enhanced 3D MR angiography
image shows typical anatomic arrangement of ascending aorta (red)
surrounded by pulmonary branches (blue) after Lecompte maneuver
arterial switch operation for dextrotransposition of great arteries in
13-year-old girl.
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Fig. 9C —Arterial switch operation (Jatene arterial switch procedure).
Axial cine MR image of same patient shown in B obtained at level of
pulmonary bifurcation during diastole shows full length of patent right and
left pulmonary arteries and typical anteroposterior position of pulmonary
trunk with respect to ascending aorta.
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Fig. 9D —Arterial switch operation (Jatene arterial switch procedure).
In this cine MR image of same patient shown in B and C, left and
right pulmonary arteries appear to be mildly compressed (arrowheads)
at same level as in C but during systole. Nonphysiologic anatomic
relationship between ascending aorta and pulmonary branches causes hemodynamic
changes in right and left pulmonary arteries. Phase velocity mapping (not
shown) did not reveal significant hemodynamic narrowing or stenosis.
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Conclusion
This article illustrates many of the complex surgical procedures performed
in patients with cyanotic congenital heart diseases. Knowledge of postsurgical
anatomy is important to avoid misdiagnosing expected anatomy as complications
on MR examinations.
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Abstract
Introduction
